Sheilas Chiari Journey

After a whole lifetime of suffering with mysterious syptoms I was diagnosised at age 42 with not only spinal and cervical spondolysis,but a rare one, so they say,Chiari MalformationI with syringomyelia.If you too are looking for answers I hope some of the following blogs and articles will help answer some of your questions and you will become a part of a very loving, caring, supportive group Chiarians. You will never be alone on your journey.

About Me

I am a wife,mother,and nana learning to live with a disorder called Chiari1 Malformation with syringomelyia & other disorders all related to the chiari.I traveled a long journey too find results and my wish is to help others find help thru my experiences.I have given up a career I worked hard at for over 3 decades, but the 1 thing that I lost, is I am unable to be the mother I had planned and should be to my daughters, and that by far is the greatest loss of all.I was happy to have validation but accepting limitations is difficult for one who was so active and independent and wanted so much more for my children, as we all do as parents. I made friendships that are more valuable to me than any pill. They make me smile, laugh, and cry,but most of all they saved me from the aloneness.My wish. after a cure is that someday I can turn to a neighbor, new friend, or even a medical professional and when I say I have Chiari the answer will not be "Chiari what is that?" Wellcome to my journey into discovering all I can about my disorders and try to bring awareness and maybe share a laugh with someone and let them know that they too will never have to travel this road alone.

Wednesday, January 28, 2009

FOR IMMEDIATE RELEASE PUBLIC SERVICE ANNOUNCEMENT
LITTLE-KNOWN SPINAL DISORDER SHOWS FEW OUTWARD SIGNS
by Virginia Bennett
Vicki is a 28 year old mother of two rambunctious children both under the age of six. She appears healthy as she enters through the doors of the gym where she works as a personal trainer. Pony-tail swinging and accompanied by the muffled sounds of her cross-trainer shoes upon the sidewalk, she greets her clients with a smile. Yet, Vicki is holding on by a thin thread, for she is fighting a number of debilitating health issues which belie her appearance of "normal." In spite of a broken spinal fusion for which she is seeking surgical help, she is also battling a rare condition known as syringomyelia which developed after a motor vehicle accident 2 years ago.
Once healthy, strong and outgoing, Josh earned his living as a hardworking power lineman. An onset of debilitating symptoms and subsequent diagnosis of syringomyelia now keeps him mostly homebound, advocating for improved working conditions for those dealing with high-intensity electrical wire atop dizzying power-poles. Vince, a renowned radio-controlled airplane competitor, now stays home to watch his children and suffers in pain after an auto accident injured his spine and left him battling syringomyelia (SM) instead of downdrafts and demanding landings. His wife works full time to pay the bills.
Keesha is also a mother of young children. In her late 20's, she has achieved three college degrees and is a qualified teacher. She should be continuing her teaching career, working in her Arizona yard or riding on amusement park rides on the weekends with her young family. Instead, she is filling out forms for Social Security Disability Insurance, traveling to hearings and doctor appointments while she faces the challenges of knock-you-to-your-knees headaches, weakness and pain throughout her body. Keesha suffers from not only syringomyelia, but also a related condition called Arnold Chiari Malformation (CM), or simply "Chiari."
Once these patients receive the diagnosis that they suffer with one or both of these incurable conditions, their lives will never be the same. They may appear "normal" to the world around them, yet they deal with neurological pain and diffuse weakness which prohibits them from living any kind of normal lifestyle.
Chiari was discovered in 1898 by Professor Hans Chiari who, during his postmortem studies in Germany, found that a certain percentage of people seemed to be born with a too-small area in the back of their skulls, thereby causing a piece of their brains to be forced down into their spinal cords. Long before the advent of MRIs and radiological imaging, Dr. Chiari found that a part of the cerebellum (which lies at the back of the head and believed to control movement) can herniate into cone-shaped "tonsils" which then fill up the hole located at the junction between the bottom of the skull and the top of the cervical spine. When the tonsils dip far enough down into this hole, the ebb and flow of cerebral spinal (CSF) fluid is obstructed.
Betsy, an artist in Pennsylvania, knows well the symptoms caused by obstructed CSF flow and the subsequent pressure upon vital nerves. She has lived with Chiari for 30 years and endured 11 surgeries. Her symptoms run the gamut from simple "pins and needles-type" tingling, numbness, impaired cognitive function, inexorable headaches which radiate out from the base of the skull toward the eyes and ears and then radiate down through the neck to the shoulders, arms and hands. Tremors are typical, along with bladder and bowel dysfunction, sleep apnea syndrome and a general weakness that defies description.
Many people are often misdiagnosed before finding out they have Chiari, a condition which afflicts women 3 to 1 over men. They are told they have multiple sclerosis, fibromyalgia, restless leg syndrome or chronic fatigue syndrome. Or, worse, in the case of women, some have been told their symptoms are attributable to PMS. Men and women alike are often told it is "all in their heads" and are given a prescription for antidepressants or psychological evaluations. Patients begin to believe those doctors respected within their community and thus question their own sanity or work ethic. Until one day, a bright, young neurologist, an older, compassionate GP or even a licensed homeopathic-practitioner recognizes the symptoms and urges the sufferer to have an MRI done of their brain.
The good news is that with the correct knowledge and expertise in Chiari, it is easy to diagnose the condition with a simple look at the patient's films. The bad news is there simply is no cure. Surgery can be performed but the best that can be promised is a slowing of the progression of symptoms. Neurosurgeons enter the back of the head at the base of the skull and remove bone and tissue to increase an opening for spinal fluid flow. Some surgeons will also cauterize the cerebellar tonsils in order to encourage the hole-plugging tissue to back away from the vital opening at the top of the spine.
Linked to Chiari Malformation is another spinal cord condition known as syringomyelia, a disease where a fluid-filled cyst or syrinx occurs within the spinal canal. CSF moves up and down through the space around the cord with each heartbeat. The fluid inside the syrinx can compress the cord out against the adjacent bony rings of the spinal vertebrae, thus compressing and damaging the nerve-laden interior of the cord . Some surgeons elect to divert any fluid from reaching the syrinx through use of a shunt inserted into the cord.
Often a syrinx is found to be caused by the obstructed CSF flow from a Chiari condition. At other times, a syrinx is brought on by head or spinal trauma such as whiplash incurred during a motor vehicle accident and no Chiari is present.
"While research into the causes of the severe, nerve-damage-related pain is progressing, the actual mechanisms involved are still poorly understood. Current medications are not effective in many cases or only partially successful in dampening down the pain and other symptoms of Chiari and syringomyelia," says Sarah Bates of Paradise, CA, herself a CM sufferer and advocate for her peers through her role as board member of the Wishes and Rainbows organization, which "educates, encourages and assists" those with CM and/or SM.
To Betsy, Vicki, Vince and others like them, the hardest things to face are often outside the doctors' offices. Families think they look fine and healthy so they must be lazy or obsessed unnecessarily with their health. Employers cannot understand why a previously outstanding employee begins to decline and cannot keep up an adequate standard of work. Insurance case managers and disability examiners cannot comprehend the pain and weakness inherent to syringomyelia and Chiari because, though these conditions are not really rare, they are not widely-known or understood. The average doctor may not encounter a single case in his career. Hence, the misdiagnoses and judgment that these sufferers must deal with in conjunction with their symptoms.
A person can be born with Chiari Malformation and lead a full life never knowing their condition exists. Such a person may only become symptomatic when trauma is experienced: an accident, fall, difficult childbirth or even a lengthy bout of sneezing. To assist with raising funds for research and helping patients find quality care and peer support, the American Syringomyelia Alliance Project (ASAP) was formed. A busy message board exists at this educational website where peers from around the world visit on a daily basis, each feeling a part of a larger family that understands his or her condition and challenges. A 20-something woman from Denmark, a mother from the UK with a 14 year old girl suffering from postoperative pain, a lonely woman in the Philippines, a young woman in Australia whose family cannot understand her pain in spite of her decompressive surgery all join in congenial visits with men and women from every state in the US. Each, in spite of their own situations, uplift others with cheerful notes or helpful advice.
"Education and awareness are key in finding treatment and a cure for those afflicted," Sarah Bates says.
For more information, visit www.asap.org and www.wishesandrainsbows.org.
Note to editor: This article may be reprinted in its entirety without permission by the author. To reach the author, please contact Virginia Bennett at virginiabennett@netscape.com.
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Tuesday, November 18, 2008

Chiari malformation is considered a congenital condition, although acquired forms of the condition have been diagnosed. A German pathologist, Professor Hans Chiari, first described abnormalities of the brain at the junction of the skull with the spine in the 1890s. He categorized these in order of severity; types I, II, III, and IV. The term “Arnold-Chiari” was latter applied to the Chiari type II malformation. These malformations, along with syringomyelia and hydromyelia, two closely associated conditions, are described below.Normal AnatomyThe cerebellum controls the coordination of motion, and is normally located inside the base of the skull, in what is referred to as the posterior fossa. Usually, the cerebellum is composed of two lateral halves or hemispheres, and a narrow central portion between these hemispheres, known as the vermis. Along the under surface of the hemispheres, there are two small protrusions called the tonsils. The fourth ventricle is a cerebrospinal fluid (CSF) filled space located in front of the cerebellum (and behind the brainstem). All of these structures are located just above the foramen magnum, the largest opening at the base of the skull through which the spinal cord enters and connects to the brainstem.PrevalenceThe prevalence in the general population has been estimated at slightly less than one in 1000. The majority of these cases are asymptomatic. Chiari malformations are often detected coincidently among patients who have undergone diagnostic imaging for unrelated reasons.Types of malformationsType I Chiari malformationThis malformation occurs during fetal development and is characterized by downward displacement by more than four millimeters, of the cerebellar tonsils beneath the foramen magnum into the cervical spinal canal. This displacement may block the normal pulsations of CSF between the spinal canal and the intracranial space. This form of Chiari malformation may be associated with syringomyelia/hydromyelia. It is diagnosed more commonly in adolescents or adults.Anomalies of the base of the skull and spine are seen in 30-50 percent of patients with Chiari I malformation. These include:Compression of the upper part of the spine into the base of the skull with resulting compression of the brainstem.Bony union of the first level of the spine (C1) to the base of the skull.Partial bony union of the first and second levels (C1 fusion to C2) of the spine.Klippel-Feil deformity (congenital union or fusion of levels of the spine within the neck with possible associated maldevelopment of levels of the cervical spine.Cervical spina bifida occulta (bony defect in the posterior part of the spine).Scoliosis – present in 16-80 percent of hydromyelia patients; especially in children with immature spines.SymptomsMany people with Chiari I malformation have no symptoms. However, any of the following symptoms may occur, alone or in combination. Some of the symptoms are related to the development of a syrinx (a fluid filled cavity in the spinal cord).Severe head and neck painAn occipital headache felt at the base of the skull that is made worse by coughing, sneezing, or strainingLoss of pain and temperature sensation of the upper torso and arms (as a result of a syrinx)Loss of muscle strength in the hands and arms (as a result of a syrinx)Drop attacks – collapsing to the ground due to muscle weaknessSpasticityDizzinessBalance problemsDouble or blurred visionHypersensitivity to bright lightsType II Chiari malformationThis malformation is characterized by downward displacement of the medulla, fourth ventricle, and cerebellum into the cervical spinal canal, as well as elongation of the pons and fourth ventricle. This type occurs almost exclusively in patients with myelomeningocele. Myelomeningocele is a congenital condition in which the spinal cord and column do not close properly during fetal development, resulting in an open spinal cord defect at birth. Other abnormalities associated with myelomeningocele include hydrocephalus, cardiovascular abnormalities, imperforate anus as well as other gastrointestinal abnormalities, and genitourinary abnormalities.SymptomsThe symptoms associated with a Chiari II malformation can also be caused by problems related to myelomeningocele and hydrocephalus. These symptoms include:Alteration in the pattern of breathing, including periods of apnea (brief periods of cessation of breathing)Depressed gag reflexInvoluntary, rapid, downward eye movementsLoss of arm strengthType III Chiari malformationThis malformation includes a form of dysraphism with a portion of the cerebellum and/or brainstem pushing out through a defect in the back of the head or neck. These malformations are very rare and are associated with a high early mortality rate, or severe neurological deficits in patients that survive. If treatment is undertaken, then early operative closure of the defect is necessitated. Hydrocephalus, which is commonly present, must also be treated through shunting.Additional severe birth defects are often present, which may require extensive treatment. Infants with Chiari III malformation may have life-threatening complications.Type IV Chiari malformationThis malformation is the most severe form and the rarest. The cerebellum fails to develop normally. There may be other associated malformations of the brain and brainstem. Most babies born with this malformation do not survive infancy.Syringomyelia/HydromyeliaWhen CSF forms a cavity or cyst within the spinal cord, it is known as syringomyelia or hydromyelia. These are chronic disorders involving the spinal cord developing, expanding or extending over time. As the fluid cavity expands, it can displace or injure the nerve fibers inside the spinal cord. A wide variety of symptoms can occur, depending upon the size and location of the syrinx. Loss of sensation in an area served by several nerve roots is one typical symptom, as is the development of scoliosis.Syringomyelia can arise from several causes. Chiari malformation is the leading cause of syringomyelia, although the direct link is not well understood. It is thought to be related to the interference of normal CSF pulsations caused by the cerebellar tissue obstructing flow at the foramen magnum.This condition can also occur as a complication of trauma, meningitis, tumor, arachnoiditis, or a tethered spinal cord. In these cases, the syrinx forms in the section of the spinal cord damaged by these conditions. As more people are surviving spinal cord injuries, increased cases of post-traumatic syringomyelia are being diagnosed.Hydromyelia is usually defined as an abnormal widening of the central canal of the spinal cord. The central canal, a very thin cavity in the middle of the spinal cord, is a remnant of normal development.SymptomsScoliosisLoss of sensitivity, especially to hot and coldMuscle weakness and spasticityLoss of bowel and bladder controlMotor impairmentChronic painHeadaches (often concurrent with the Chiari malformation)DiagnosisThere are several tests that can help diagnose and determine the extent of Chiari malformation and syringomyelia.Brainstem auditory evoked potential (BAER): An electrical test to examine the function of the hearing apparatus and brainstem connections. This is used to determine if the brainstem is working properly.Computed tomography scan (CT or CAT scan): A diagnostic test that creates an image by computer reconstruction of x-rays; it is particularly good at defining the size of the cerebral ventricles and showing an obvious blockage. It is less effective for analysis of the posterior fossa or the spinal cord.Magnetic resonance imaging (MRI): A diagnostic test that produces three-dimensional images of body structures using magnetic fields and computer technology. It can provide an accurate view of the brain, cerebellum and the spinal cord, is very good at defining the extent of malformations, and distinguishing progression. The MRI provides more information than the CT scan when analyzing the back of the brain and spinal cord, and is usually the preferred test.Myleogram: An x-ray of the spinal canal following injection of a contrast material into the CSF space; can show pressure on the spinal cord or nerves due to malformations. This test is performed less frequently now.Somatosensory evoked potentials (SSEP): An electrical test of the nerves involved in sensation, which gives some information about peripheral nerve, spinal cord, and brain function.TreatmentTreatment of Chiari malformations and syringomyelia is very dependent on the exact type of malformation, as well as progression in anatomy changes or symptoms.Chiari I malformations that are asymptomatic should be left alone. There is no indication for "prophylactic" surgery on these. If the malformation is defined as symptomatic, or is causing a syrinx, treatment is usually recommended.Chiari II malformations are treated if the patient is symptomatic, and physicians have determined that there are no complications from hydrocephalus. In some patients, consideration of a tethered cord is also explored. In many infants who become symptomatic from a Chiari II malformation, the symptom onset and progression are severe and rapid, and this requires an urgent or emergency approach.SurgerySurgical treatment of these malformations depends on the type of malformation. The goal of surgery is to relieve the symptoms, or stop the progression of the syrinx or symptoms.Chiari I malformations may be treated surgically with only local decompression of the overlying bones, decompression of the bones and release of the dura (a thick membrane covering the brain and spinal cord), or decompression of the bone and dura and some degree of cerebellar tissue resection.Decompression is performed under general anesthesia. It consists of removing the back of the foramen magnum and often the back of the first few vertebrae to the point where the cerebellar tonsils end. This provides more space for the brainstem, spinal cord, and descended cerebellar components. A tissue graft is often spliced into this opening to provide even more room for the unimpeded passage of CSF. Occasionally, the cavity within the spinal cord resulting from hydromyelia can be drained with a diverting shunt tube. This tube can divert the fluid from inside the spinal cord to outside the cord, or be directed to either the chest or abdominal cavity. These procedures can be done together or separately.Chiari II decompression is treated similarly, but is usually restricted to decompressing the tissues in the spinal canal and leaving the back of the skull alone.The goal of Chiari surgery is:Optimal decompression of nerve tissueReconstruction of normal CSF flow around and behind the cerebellum .OutcomeThe benefits of surgery should always be weighed carefully against its risks. Although some patients experience a reduction in their symptoms, there is no guarantee that surgery will help every individual. Nerve damage that has already occurred usually cannot be reversed. Some surgical patients need repeat surgeries, while others may not achieve symptom relief.Glossary of TermsAperture: An opening or space between bones or within a bone.Basilar impression: When the base of the skull is pushed “up” into the skull.Brainstem: The portion of the brain connecting the spinal cord with the midbrain/cortex, which houses the controls of many basic functions such as breathing, swallowing, eye movement.Cavity: An open area or space, such as a sinus within a bone.Central canal: A small tubular cavity in the center of the spinal cord which is normally not dilated.Cerebellum: The portion of the brain which is in the posterior fossa. It is involved in coordination of movements.Cranium: All of the bones of the skull except for the mandible.Dura: The covering over the surface of the brain and spinal cord.Dysraphism: Developmental abnormalities in the midline region of the back, with incomplete fusion or malformation of a seam, such as in all forms of spina bifida.Foramen magnum: The opening at the base of the skull through which the spinal cord passes.Hydrocephalus: A condition in which excess cerebrospinal fluid (CSF) builds up within the ventricles (fluid-containing cavities) of the brain and may increase pressure within the head.Medulla oblongata: Lowest part of the brainstem, located just above the spinal cord, at the exit from the skull. Provides control for breathing and heart function.Neurocranium: Referring to the braincase of the skull.Pons: Band of nerve fibers linking the medulla oblongata and the cerebellum with the midbrain.Posterior fossa: The cavity in the back part of the skull which contains the cerebellum, brainstem, and cranial nerves 5-12.Shunt: A tube which drains spinal fluid from one space to another body cavity.Spasticity: Increased tightness or tone in the arms and/or legs, making one less flexible and possibly causing the arms and/or legs to stiffen.Splanchocranium: Referring to the facial bones of the skull.Suture: The saw-like edge of a cranial bone that serves as joint between bones of the skull.Syrinx (syringomyelia, hydromyelia): These terms all refer to a fluid filled cavity in the spinal cord.Tonsils: The portion of the cerebellum that protrudes into the spinal canal, which may become elongated.Vallecula (of cerebellum): The longitudinal hollow on the inferior surface of the cerebellum, between the hemispheres, in which the medulla oblongata rests.Ventricles: Fluid filled cavities within the brain. The spinal fluid is produced by a gland in the ventricle (about two pints each day) and circulates through the ventricles and over the surface of the brain into the veins. If there is a block in the system, the fluid can build up and cause hydrocephalus.Vermis: The narrow middle zone between the two hemispheres of the cerebellum; the portion projecting above the level of the hemispheres on the upper surface is called the superior vermis; the lower portion, sunken between the two hemispheres and forming the floor of the vallecula, is the inferior vermis.

Thursday, September 18, 2008

I'm glad to see that you are awake! This is your brain talking. I had to find some way to communicate with you. I feel like I barely survived WWIII and am still not quite all in one piece. That's why I need you. I need you to take care of me.

As time passes and you and I feel better and better, people, even doctors, will tell you that we are fine, "it's time to get on with life." That sounds good to me and probably even better to you. But before you go rushing back out into that big wide world, I need you to listen to me, really listen. Don't shut me out. Don't tune me out. When I'm getting into trouble I'll need your help more than I ever have before.

I know that you want to believe that we are going to be the same. I'll do my best to make that happen. The problem is that too many people in our situation get impatient and try to rush the healing process; or when their brains can't fully recover they deny it and, instead of adapting, they force their brains to function in ways they are no longer able too. Some people even push their brains until they seize, and worse... I'm scared. I'm afraid that you will do that to me. If you don't accept me I am lost. We both will be lost.

How can I tell you how much I need you now? I need you to accept me as I am today... not for what I used to be, or what I might be in the future. So many people are so busy looking at what their brains used to do, as if past accomplishments were a magical yardstick to measure present success or failures, that they fail to see how far their brains have come. It's as if here is shame, or guilt, in being injured. Silly, huh?

Please don't be embarrassed or feel guilt, or shame, because of me. We are okay. We have made it this far. If you work with me we can make it even further. I can't say how far. I won't make any false promises. I can only promise you this, that I will do my best.

What I need you to do is this: because neither of us knows how badly I've been hurt (things are still a little foggy for me), or how much I will recover, or how quickly, please go s-l-o-w-l-y when you start back trying to resume your life. If I give you a headache, or make you sick to your stomach, or make you unusually irritable, or confused, or disoriented, or afraid, or make you feel that you are overdoing it, I'm trying to get your attention in the only way I can. Stop and listen to me.

I get exhausted easily since being hurt, and cannot succeed when overworked. I want to succeed as much as you do. I want to be as well as I can be, but I need to do it at a different pace than I could before I got hurt. Help me to help us by paying attention and heeding the messages I send to you.

I will do my part to do my very best to get us back on our feet. I am a little worried though that if I am not exactly the same... you will reject me and may even want to kill us. Other people have wanted to kill their brains, and some people have succeeded. I don't want to die, and I don't want you to die.

I want us to live, and breath and be, even if being is not the same as it was. Different may be better. It may be harder too, but I don't want you to give up. Don't give up on me. Don't give up on yourself. Our time here isn't through yet. There are things that I want to do and I want to try, even if trying has to be done in a different way. It isn't easy. I have to work very hard, much harder, and I know that you do too. I see people scoff, and misunderstand. I don't care. What I do care about is that you understand how hard I am working and how much I want to be as good as I can be, but I need you to take good care of us, as well as you can do that.

Don't be ashamed of me. We are alive. We are still here. I want the chance to try to show you what we are made of. I want to show you the things that are really important in life. We have been given another chance to be better, to learn what is really important. When it is finally time for our final exit I would like to look back and feel good about what we made of us and out of everything that made up our life, including this injury. I cannot do it without you. I cannot do it if you hate me for the way being injured has affected me and our life together. Please try not to be bitter in grief. That would crush me.

Please don't reject me. There is little I can do without you, without your determination to not give up. Take good care of us and of yourself. I need you very much, especially now.

Saturday, September 13, 2008

I just currently read this article again after comming face to face with a women that gave me a look to kill, and I was having a bad day with pain anyway, so I shouted out "do you have any idea what chronic pain is?" Look it up if you don't and keep your dirty looks to yourself." I felt bad because I am usualy not an angry person but was I am totaly feed up with having to explain my life and what a day with pain does to me and my whole famly and that was the last straw. If any of you have not read this article by an angel :Lisa Copen, you need to, especialy when this is chronic illness awareness month and also Chiari awareness month which is the disorder that is near and dear to me, since I have this disorder. So next time when you see someone that may look Ok on the outside remember there are many illnesses that are invisible and they deserve the right to park there too, keep your looks friendly because you never know when you or someone you love will become ill and it won't show to others, how would you want them to be treated?I don't remember the whole saying or who said it, but you don't know a man/women unless you've walked a day in their shoes. That's powerful and true..